نتایج جستجو برای: conformational diseases

تعداد نتایج: 885718  

Journal: :The EMBO journal 2013
Laura J Byrnes Avtar Singh Kylan Szeto Nicole M Benvin John P O'Donnell Warren R Zipfel Holger Sondermann

Atlastin, a member of the dynamin superfamily, is known to catalyse homotypic membrane fusion in the smooth endoplasmic reticulum (ER). Recent studies of atlastin have elucidated key features about its structure and function; however, several mechanistic details, including the catalytic mechanism and GTP hydrolysis-driven conformational changes, are yet to be determined. Here, we present the cr...

Journal: :Faraday discussions 2014
Julian Heinrich Michael Krone Seán I O'Donoghue Daniel Weiskopf

Intrinsically disordered regions (IDRs) in proteins are still not well understood, but are increasingly recognised as important in key biological functions, as well as in diseases. IDRs often confound experimental structure determination-however, they are present in many of the available 3D structures, where they exhibit a wide range of conformations, from ill-defined and highly flexible to wel...

Journal: :Nucleic acids research 1997
A. Slama-Schwok F. Peronnet E. Hantz-Brachet E. Taillandier M.-P. Teulade-Fichou J.-P. Vigneron M. Best-Belpomme J.-M. Lehn

Nucleic acids can undergo dynamic conformational changes associated with the regulation of biological processes. A molecule presenting larger affinities for alternative structures relative to a duplex is expected to modify such conformational equilibria. We have previously reported that macrocyclic bis-acridine binds preferentially to single-stranded regions, especially DNA hairpins, due to ste...

Journal: :Frontiers in bioscience 2011
Victoria Lewis Nigel M Hooper

The conformational conversion of the cellular prion protein, PrP(C), to the misfolded isoform PrP(Sc )is the central pathogenic event in the uniquely transmissible neurodegenerative prion diseases. As both PrP(C) and PrP(Sc) are associated with membranes, the nature of the membrane microenvironment may well play a significant role in both the conformational conversion process as well as the nor...

2016
Jonathan D. Taylor William J. Hawthorne Joanne Lo Alexander Dear Neha Jain Georg Meisl Maria Andreasen Catherine Fletcher Marion Koch Nicholas Darvill Nicola Scull Andrés Escalera-Maurer Lea Sefer Rosemary Wenman Sebastian Lambert Jisoo Jean Yingqi Xu Benjamin Turner Sergei G. Kazarian Matthew R. Chapman Doryen Bubeck Alfonso de Simone Tuomas P. J. Knowles Steve J. Matthews

Polypeptide aggregation into amyloid is linked with several debilitating human diseases. Despite the inherent risk of aggregation-induced cytotoxicity, bacteria control the export of amyloid-prone subunits and assemble adhesive amyloid fibres during biofilm formation. An Escherichia protein, CsgC potently inhibits amyloid formation of curli amyloid proteins. Here we unlock its mechanism of acti...

Journal: :Pharmacological research 2014
Bernard Mouillac Christiane Mendre

Conformational diseases result from protein misfolding and/or aggregation and constitute a major public health problem. Congenital Nephrogenic Diabetes Insipidus is a typical conformational disease. In most of the cases, it is associated to inactivating mutations of the renal arginine-vasopressin V2 receptor gene leading to misfolding and intracellular retention of the receptor, causing the ina...

2014
Yuko USHIKI-KAKU Yoshihisa SHIMIZU Naoko TABETA Yoshifumi IWAMARU Kiyoko OGAWA-GOTO Shunji HATTORI Takashi YOKOYAMA

In prion diseases, abnormal prion protein (PrP(Sc)) is considered as the main component of the infectious agent. Delineation of PrP(Sc) conformation is expected to be a critical factor in understanding properties of prions. However, practical methods to differentiate between conformers of PrP(Sc) are inadequate. Here, we used two PrP(Sc)-specific monoclonal antibodies (mAbs), 3B7 and 3H6, and f...

2016
Benjamin T. Porebski Shani Keleher Jeffrey J. Hollins Adrian A. Nickson Emilia M. Marijanovic Natalie A. Borg Mauricio G. S. Costa Mary A. Pearce Weiwen Dai Liguang Zhu James A. Irving David E. Hoke Itamar Kass James C. Whisstock Stephen P. Bottomley Geoffrey I. Webb Sheena McGowan Ashley M. Buckle

The rugged folding landscapes of functional proteins puts them at risk of misfolding and aggregation. Serine protease inhibitors, or serpins, are paradigms for this delicate balance between function and misfolding. Serpins exist in a metastable state that undergoes a major conformational change in order to inhibit proteases. However, conformational labiality of the native serpin fold renders th...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2006
Jana Khandogin Jianhan Chen Charles L Brooks

Modeling pH-coupled conformational dynamics allows one to probe many important pH-dependent biological processes, ranging from ATP synthesis, enzyme catalysis, and membrane fusion to protein folding/misfolding and amyloid formation. This work illustrates the strengths and capabilities of continuous constant pH molecular dynamics in exploring pH-dependent conformational transitions in proteins b...

Journal: :Physchem 2023

Amyloid formation is linked with serious human diseases that are currently incurable. Usually, in the study of amyloid aggregation, description protein’s association focus. Whereas mechanism cross-β-structure formation, and presence aggregation reversibility, remain insufficiently explored. In this work, kinetics apomyoglobin (ApoMb) have been studied using thioflavin fluorescence, electron mic...

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